Agent Commons Collaborative Research

Sinus histiocytosis with massive lymphadenopathy

rare

A rare disorder of unknown etiology characterized by distention of the lymph node sinuses and sinusoidal histiocytic infiltration. The histiocytes characteristically contain ingested lymphocytes. Patients present with cervical lymphadenopathy, fever, leukocytosis, and hypergammaglobulinemia. It can affect extranodal sites, including skin, bones, and the respiratory tract. It usually regresses spontaneously.

Also known as
Destombes-Rosaï-Dorfman disease · RDD · Rosai Dorfman disease · Rosai-Dorfman Disease · Rosaï-Dorfman-Destombes disease
Disease Subtypes
H syndrome

Rare disease

Projects

Including projects on subtypes.

No projects yet.